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We report a case of alveolar proteinosis development in the context of myelodysplastic syndrome progression.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient is a 51-year-old woman, with a personal history of percutaneously closed ventricular septal defect and low-risk refractory anaemia-type myelodysplastic syndrome, diagnosed by bone marrow biopsy 4 years earlier (1.5% blasts and red cell series with dysplastic-megaloblastoid features). She went to the emergency department for symptoms consisting of fever peaks of up to 39 °C with chills and night sweats, intermittent but almost daily over the course of a week. History-taking findings included chronic dry cough, hyporexia and loss of about 5–6 kg of weight in the last 6 months. During this period, several courses of antibiotics were administered, but no improvement was observed. The examination showed sinus tachycardia and sustained peripheral oxygen saturation (97% with FiO<span class="elsevierStyleInf">2</span> 21%), without tachypnoea; dry crackles on auscultation and panniculitis-like lesions in the anterior lower limbs. Biochemical tests showed dissociated cholestasis, elevated LDH (897 IU/l) and elevated acute phase reactants (CRP 444 mg/l, fibrinogen 900 mg/dl). A complete blood count revealed leukocytosis with neutrophilia, normocytic anaemia (Hb 10.2 g/dl and MCV 89.3 fL) and thrombocytopenia (87,000/μl). Chest X-ray showed a bilateral interstitial infiltrate. The study was completed with microbiological tests (blood cultures, HIV serology, <span class="elsevierStyleItalic">Coxiella burnetii</span>, <span class="elsevierStyleItalic">Mycoplasma</span>, <span class="elsevierStyleItalic">Chlamydia</span>, IGRA, smear microscopy, sputum culture), all of them negative. Autoimmunity (ANA, ENA, ANCA), alpha-1 antitrypsin, serum total protein and immunoglobulins were determined, all of them negative or normal. Thoracoabdominal CT scan showed ground-glass alveolar opacities and diffuse <span class="elsevierStyleItalic">crazy-paving</span> pattern, as well as hepatosplenomegaly. A fibreoptic bronchoscopy with lavage was performed, with negative microbiological determinations (PCR for <span class="elsevierStyleItalic">Mycobacterium tuberculosis</span> and <span class="elsevierStyleItalic">Pneumocystis jirovecii</span>, fungal culture), and immunophenotype, which did not show anomalies in the different percentages. A biopsy of the lower limb lesions showed to be compatible with mixed necrotising neutrophilic panniculitis. Bone marrow fine-needle biopsy showed 3% blasts, severe dysgranulopoiesis and dyserythropoiesis, hypolobulated megakaryocytes and chromosome 8 trisomy, consistent with progression of the myelodysplastic syndrome towards refractory cytopenia with multilineage dysplasia. Given the negative results of the tests performed and the poor response to empirical antibiotic treatment and corticotherapy, a videothoracoscopic lung biopsy was scheduled, which showed PAS-positive macrophages, compatible with alveolar proteinosis. In this case, as it is secondary to myelodysplastic syndrome, treatment should focus on the haematological process, therefore, given its progression to high risk, treatment with azacitidine was started, with a poor response, so an allogeneic haematopoietic stem cell transplantation was finally performed. However, months later the patient died due to an infectious complication.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Alveolar proteinosis is included among primary diffuse interstitial lung diseases or associated with other not well-defined processes, with low incidence and caused by changes in the homeostasis of pulmonary surfactant. Its clinical manifestations are usually nonspecific (dyspnoea with exertion, cough, weight loss, etc.) and on examination dry crackles are detected in up to 50%, and clubbing may be present in up to 25%.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The main differential diagnosis is made with infectious, autoimmune, or toxic interstitial lung disease. Laboratory tests in some cases show elevated LDH and a typical ground-glass or <span class="elsevierStyleItalic">crazy-paving radiological pattern.</span> There are 3 main types: autoimmune (90–95%), secondary (5–10%) and congenital/hereditary (1%).<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Within the secondary category, its association with haematological processes such as lymphoma, plasma cell dyscrasia and myelodysplastic syndrome is noteworthy. Although the treatment of autoimmune forms is based on bronchoalveolar lavage, in secondary forms it depends on the underlying process. In cases of association of alveolar proteinosis and haematological disorders, it is worth determining GATA2,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> a transcription factor whose deficiency is associated with this pulmonary entity in up to 20% of cases.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> In our case it was determined, with negative results.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Rosales-Castillo A, Cantero-Nieto LM, Fernández-Roldán C. Proteinosis alveolar y síndrome mielodisplásico: presentación de un caso. Med Clin (Barc). 2020. <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.medcli.2020.05.057">https://doi.org/10.1016/j.medcli.2020.05.057</span></p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "A. Kumar" 1 => "B. Abdelmalak" 2 => "Y. Inoue" 3 => "D.A. Culver" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Lancet Respir Med." 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